Systemic sclerosis (symptoms, diagnosis, treatment, classification)

1. Big picture

Systemic sclerosis (SSc), or scleroderma, is a chronic systemic autoimmune disease characterized by a triad:

microvascular injury + immune activation + fibrosis of skin and internal organs

Classic exam pattern:

Raynaud phenomenon + puffy fingers/sclerodactyly + abnormal nailfold capillaries + anti-centromere or anti-Scl-70 antibodies + esophageal dysmotility/lung disease.

The main causes of death are usually interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). The emergency you must never miss is scleroderma renal crisis.